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Reconstruction of the orbital cavity after carcinologic exenteration in a pediatric population: 13 cases.
Paige McLean, MD1 Samuel Lance, MD1 Jean Francois Honart, MD2 Natacha Kadlub, MD3 Frederic Kolb, MD1 1 Department of Surgery, Division of Plastic Surgery, UC San Diego, San Diego, CA 2 Department of Plastic Surgery, Institut Gustave Roussy, Paris, France 3 Department of Maxillofacial Surgery, Hopital Universitaire des Enfants Maladies Necker, Paris, France

Background: Indications of orbital exenteration are rare in children and mostly related to carcinologic reasons. Retinoblastoma represents an indication for limited enucleation that requires no reconstruction or at most a skin graft. Rehabilitation of the orbital socket in these cases is straight forward and is performed with a prosthesis, leading to a good cosmetic result. Exenterations require more complex reconstructive techniques which combine flaps, implants, and epithesis. The indications for orbital exenterations are rare and as such publications present in the literature today include case reports or limited series. This has prevented the development of consensual guidelines for management of these defects that take into account the long-term effects of radiotherapy and facial growth. We present our experience of 13 cases with a mean follow-up of 11.35 years.

Methods: Cases of exenterations at 3 institutions (Gustave Roussy Paris France, Necker Hospital Paris France and Rady Children's Hospital San Diego USA) were reviewed. Thirteen children matched inclusion criteria. They were 9 males and 4 females, with a median age at surgery of 7.18 years. The mean follow-up is 11.35 years. Rhabdomyosarcoma (11 cases) was the most frequent histology. Squamous cell carcinoma and retinoblastoma were the other histologic sub-types. Special attention was given to the extent of resection using Cinar's classification, the type of reconstruction, the carcinologic outcome and the technique of orbital socket rehabilitation.

Results: Type III and IV resections represented over half of the cases. Complete or partial eyelid preservation was performed in 9 cases. A locoregional flap was used in 7 cases (temporal muscle, temporoparietal fascia, or a combination of the two). Six free flaps were performed (latissimus dorsi and scapulo-dorsal). Eight children had radiotherapy. Ten children are alive and have been followed, on average 12.23 years. Six children required multiple surgeries and two required a second free flap in order to obtain an acceptable orbital cavity rehabilitation. In all cases, the quality of tissues filling the orbital cavity provided a stable orbital rehabilitation. No ulceration or fistulas were experienced. Four children have reached adulthood, presenting a definitive result. Three have an eye prosthesis and one has an implant-born epithesis. Cosmetic result has been judged fair in 2 and poor in 2. Four of the patients in childhood have an eye prosthesis with cosmetic results judged as fair. In one, implants have been placed, and the epithesis is not yet manufactured. One hasn't started his rehabilitation and results cannot be assessed yet in 3.

Conclusion: Reconstruction of the orbital cavity after wide exenteration requires a locoregional or free flap. It is necessary to provide stable tissues of good quality in order to obtain an acceptable orbital socket rehabilitation. Long-term cosmetic results reported thus far have been suboptimal, mainly due to poor eyelid shape. An algorithm helping to determine when the eyelids can be preserved or when they need to be sacrificed can improve the long-term cosmetic outcomes.

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